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目的 :了解外胚层发育不良综合征(ectodermal dysplasia,ED)患者的颌面部发育状况,为临床治疗提供参考。方法:收集2013年6月—2016年10月收治的48例ED患者的临床信息和头颅侧位X线片。将患者分为2组:第1组23例,除缺牙外,累及≥2种外胚层组织;第2组25例,除缺牙外,仅累及1种外胚层组织。比较2组病例的缺牙数及颌面骨形态影像学参数的差异。采用SPSS 17.0软件包对数据进行单样本t检验和成组t检验。结果:第1组缺牙数显著多于第2组(22.3±5.8∶12±6.6,P<0.05)。在女性患者中,第1组滞留乳牙数显著多于第2组(8±4∶2.4±2.7,P<0.05)。2组患者的上颌骨长度减少(ANS-Ptm减小)且位置后缩(S-Ptm、SNA、NA-PA减小),下颌骨前突(NP-FH增大)、颏部前突(Y轴角减小),下颌骨长度(Co-Po)及位置(S-Co)相对正常,骨性Ⅲ类错(ANB<0),全面高降低(NMe减小)。在男性患者中,第1组较第2组的SNA、NA-PA、ANS-Ptm、S-Ptm、Y轴角显著减小(P<0.05),NP-FH显著增大(P<0.05)。结论:ED患者颌面骨形态表现为上颌骨长度减少及位置后缩,下颌骨长度和位置相对正常,下颌骨前突、颏部前突、骨性Ⅲ类错和全面高降低。ED患者颌面骨形态异常的严重程度与缺失的恒牙数呈正相关,滞留的乳牙可能会促进颌面骨发育。
Objective: To understand the maxillofacial development in patients with ectodermal dysplasia (ED) and provide a reference for clinical treatment. Methods: The clinical data and cranial X-ray of 48 ED patients who were admitted from June 2013 to October 2016 were collected. The patients were divided into two groups: Group 1 23 cases, excluding edentulous, involving more than 2 kinds of ectodermal tissue; Group 2 25 cases, except for edentulous, involving only one kind of ectodermal tissue. The differences of the numbers of missing teeth and maxillofacial bone morphological parameters between the two groups were compared. SPSS 17.0 software package was used to perform a one-sample t-test and a t-test on the data. Results: The number of missing teeth in group 1 was significantly more than that in group 2 (22.3 ± 5.8:12 ± 6.6, P <0.05). In female patients, the number of retained teeth in group 1 was significantly more than that in group 2 (8 ± 4: 2.4 ± 2.7, P <0.05). The maxillary length was decreased (ANS-Ptm decreased) and the position was reduced (S-Ptm, SNA, NA-PA decreased), mandibular prognathism (NP- FH increased), chin forearm (Y-axis angle decreases), mandibular length (Co-Po) and position (S-Co) are relatively normal, skeletal Class Ⅲ error (ANB <0), overall high decrease (NMe decrease). In male patients, the axial angles of SNA, NA-PA, ANS-Ptm, S-Ptm and Y were significantly decreased (P <0.05) and NP-FH was significantly increased . Conclusion: The morphology of maxillofacial bone in patients with ED showed reduction of maxillary length and posterior retraction, relatively normal length and position of mandible, mandibular prognathism, chin anterior protrusion, and complete osteopenia. The severity of maxillofacial abnormalities in ED patients was positively correlated with the number of missing permanent teeth. The retained primary teeth may promote maxillofacial development.